Investigation of Inherited Chromosomally Integrated Human Herpesvirus-6A+and-6B+in a Patient with Ulipristal Acetate-Induced Fulminant Hepatic Failure

Inherited chromosomally integrated (ici) human herpes virus 6 (HHV-6) is estimated to occur in 0.6-2.7% of people worldwide. HHV-6 comprises two distinct species: HHV-6A and HHV-6B. Both HHV-6A and HHV-6B integration have been reported. Several drugs are capable of activating iciHHV-6 in tissues, the consequences of which are poorly understood. We report herein a case of a woman with iciHHV-6A+ and iciHHV-6B+, who developed ulipristal acetate (a selective progesterone receptor modulator)-induced fulminant hepatic failure that required liver transplantation. We confirmed the presence of ~one copy per cell of both HHV-6A and HHV-6B DNA in her hair follicles using multiplex HHV-6A/B real-time PCR and demonstrated the Mendelian inheritance of both iciHHV-6A and iciHHV-6B in her family members over three generations. Because of the rarity of this presentation, we discuss herein the possible links between reactivated HHV-6 from iciHHV-6A and/or iciHHV-6B and adverse drug reactions, suggesting that iciHHV-6 could be screened before the introduction of any hepatotoxic drugs to exclude HHV-6 active disease or combined idiosyncratic drug-induced liver injury in these patients.

Automated summary

This article reports a case of a patient with iciHHV-6A and iciHHV-6B who developed acute fulminant hepatic failure after using ulipristal acetate, and the Mendelian inheritance of the virus was confirmed through family studies. Due to the rarity of this presentation, the possible links between iciHHV-6 and adverse drug reactions were discussed, and it was suggested to screen for iciHHV-6 before using hepatotoxic drugs.